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Ocular Inflammatory Changes in Established Multiple Sclerosis

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Ocular Inflammatory Changes in Established Multiple Sclerosis J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.52.12.1360 on 1 December 1989. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry 1989;52:1360-1363 Ocular inflammatory changes in established multiple sclerosis E M GRAHAM, D A FRANCIS, M D SANDERS, P RUDGE From the National Hospitalfor Nervous Diseases, Queen Square, London suMMARY Fifty consecutive patients with clinically definite multiple sclerosis were studied to assess the prevalence of concomitant uveitis. Asymptomatic ocular inflammatory changes were found in nine patients (18%) and appeared to show a positive correlation with severe and progressive disease. Conversely uveitis was uncommon in the presence of established optic atrophy which suggests a negative influence on its pathogenesis. In the absence of optic atrophy inflammatory changes in the eye may be a valuable index of disease activity. Ocular inflammatory changes have been a recognised Patients with currently progressive MS had shown either a occurrence in multiple sclerosis (MS) for many years,' progressive evolution of disease from onset or had entered a although their pathogenetic significance remain un- progressive phase following initial remissions. guest. Protected by copyright. certain. Recent studies have found bet- All fifty patients had a full ophthalmological examination. associations This included assessment ofcorrected visual acuity (Snellen), ween retinal vascular abnormalities and both the later colour vision (Ishihara plates), visual fields (Bjerrum screen); development of MS in patients presenting with slit-lamp examination and direct and indirect ophthalmo- isolated optic neuritis (ON)2 and neurological disease scopy. Fluorescein angiography was performed only when activity in patients with established MS.3 clinically indicated. Ocular inflammation (uveitis) was In this study we have sought the prevalence of defined by the presence of inflammatory cells in the vitreous ocular inflammatory changes (uveitis) in patients with with either sheathing ofperipheral retinal vessels and/or focal clinically definite MS and correlated their presence cuffing of retinal veins (periphlebitis). with clinical course and disease severity. Our findings may shed further light on their occurrence and patho- Results genesis. Neurologicalfeatures Patients and methods The neurological features are summarised in table 1. The mean duration ofMS for the group as a whole was We studied consecutive patients with clinically definite MS 12-3 years (range 0-5-38 years); fifty per cent of but without preceding ocular disease (see below),4 who were patients had had neurological symptoms 10 admitted to one neurological firm at the National Hospital, for years Queen Square, London. Patients with a past history ofocular or more. Thirty four patients (68%) had remittent disease, other than optic neuritis, or systemic illness disease and in 16 patients (32%) the disease was The age at onset and disease associated with ocular abnormalities were excluded. A total currently progressive. http://jnnp.bmj.com/ of 50 patients was collected, 27 were female and 23 male. Their mean age at presentation of first neurological symp- Table 1 Clinicalfeatures of50 consecutive patients toms was 28-3 years (range 16-51 years). with MS Patients were categorised according to the severity (mild, moderate or severe) and clinical course (remittent or cur- Disease rently progressive) oftheir disease. Patients with mild disease Sex Age at duration had a score of < 3 on the Kurtzke disability scale,5 moderate onset (mean disease 3 5-6 and severe disease > 6 at the time ofassessment. M (%)F(%) (mean yrs) yrs) Disease course: on September 24, 2021 by Remittent(68%) 38-2 61 8 280 11-2 Correspondence to: Dr D A Francis, National Hospital for Nervous Progressive (32%) 62 5 37-5 28-9 14-7 Diseases, Maida Vale, London W9 ITL, United Kingdom. Disease severity: Mild/Mod (54%) 48-2 51-8 29-3 9-6 Received 10 March 1989 and in revised form 9 June 1989. Severe (46%) 39-1 60 9 27-1 15 6 Accepted 17 July 1989 1360 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.52.12.1360 on 1 December 1989. Downloaded from Ocular inflammatory changes in established multiple sclerosis 1361 Table 2 Neurological status ofpatients with ocular qnjiammation (uveitus) ;A+: /o ...............J~! Neurological status Uveitis % Overall (50) 18-0 (9) Remittent (34) 11-8 (4) Progressive (16) 31-3 (5) Mild (12) 8-3 (1) Moderate (15) 20-0 (3) Severe (23) 21-7 (5) Disease duration: >1O years (25) 28-0 (7) <1O years (25) 8-0 (2) ) = Number of patients. duration were similar in both groups, although females tended to follow a remittent course and males a more progressive evolution. Twenty seven patients (54%) had disease of mild to moderate severity; 23 patients (46%) severe MS. Females predominated in the severe category but overall had had their disease for a longer period. guest. Protected by copyright. Ophthalmoscopicfeatures Nine patients (18%; five males, four females) had evidence of ocular inflammation (venous sheathing in three; focal venous cuffing in four) at the time of ophthalmoscopic examination. Their neurological status is summarised in table 2; ocular inflammatory changes were present in 31 2% of patients with progressive disease (five patients), 21 7% of patients with severe MS (five patients) and 28% of patients with disease of more than 10 years duration (seven patients). In eight patients the inflammatory changes were present in both eyes. In one patient it was unilateral; the other eye, showing marked optic atrophy secon- dary to previous trauma, had no signs of uveitis. This patient underwent fluorescein angiography (fig 1) and further electrodiagnostic studies. The visual evoked Fig 1 (a top). Latefluorescein angiogram ofright eye responses (VER) from both eyes were significantly showing dilated retinal veins with perivascular leakage delayed (right eye, 145 msec; left eye, 175 msec; normal (arrow). (b bottom). Latefluorescein angiogram ofleft eye http://jnnp.bmj.com/ range 95-117 msec). The electro-oculogram (EOG) which does not show any evidence ofvascular leakage. and the electroretinogram (ERG) also showed bi- lateral abnormalities although conversely the right eye was marginally worse than the left. The EOG light-rise (35%) compared to 46/81 (55%) of unaffected eyes on the right was 200% and on the left 220% (NR 250- (chi-square = 2.6). 300%); the averaged cone ERG was 5-8 pv on the right and 7O0 iv on the left (NR 8-9 pv). Discussion Visual function appeared better preserved in on September 24, 2021 by patients with uveitis thus visual acuity was 6/9 or The combination ofretinal vascular abnormalities and better in all 17 eyes with active inflammation com- neurological disease is seen in three distinct groups of pared to 80% of unaffected eyes; colour vision was patients. Firstly, those that Rucker' identified with impaired in only 1/17 "affected" eyes compared to MS who have established neurological disease with no (75%) ofthe remainder and optic atrophy was present symptoms referable to ocular inflammation, but in in only six of 17 eyes showing inflammatory changes whom routine ophthalmological examination reveals J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.52.12.1360 on 1 December 1989. Downloaded from 1362 Graham, Francis, Sanders, Rudge cells in the vitreous, peripheral venous sheathing and/ For example patients with Behcet's disease who or focal cuffing of the retinal veins. Secondly patients experience recurrent uveitis with retinal vascular present complaining of "floaters" across their vision occlusion will eventually develop optic atrophy; at this which are caused by inflammatory cells in the vitreous; stage the ocular disease process is arrested and uveitis these patients also have sheathing of their peripheral never recurs (EMG unpublished data). retinal vessels and diffuse vascular leakage demon- The mechanism of this phenomenon is unclear but strated on fluorescein angiography. A recent study of there are at least two possible explanations. One is to 65 patients with isolated retinal vasculitis has revealed assume that severe optic atrophy reduces retinal that 10% later developed MS.6 The third group of vascularity and lessens the opportunity for immuno- patients have symptomatic ocular and neurological logical reactions to take place at the vascular endo- disease at presentation. The eye disease is often termed thelium. The second is that, if a component of CNS "Eales disease"7 and is characterised by severe peri- tissue is the prime immunological target, the inflam- phlebitis, retinal vein occlusion leading to neovascu- matory reaction would be expected to subside with its larisation and vitreous haemorrhage. The neuro- progressive loss. The electrodiagnostic and fluorescein logical component is usually monophasic and most studies in our patient with traumatic left optic atrophy often consists of a spastic paraparesis.89 Multiple confirmed that optic nerve damage was the predomin- sclerosis is a rare cause of this combination whereas ant pathological feature in the left eye whereas retinal tuberculosis, sarcoidosis or the systemic vasculitides damage was more pronounced in the right eye. Retinal are frequently associated. photoreceptor damage is known to occur as a result of This study is confined to the first group of patients retinal vasculitis'3 and this is borne out by the asym- defined above in which we have found an 18% metrical ERG responses we obtained; the ERG from prevalence of
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